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Angioedema. Overview. Self-Limited, subcutaneous edema resulting from increased vascular permeability Dilation of venules and capillaries Different from urticaria Limited to the superficial dermis Generally resolves over 24-48 hours Mast Cell / Kinin related etiologies

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overview
Overview
  • Self-Limited, subcutaneous edema resulting from increased vascular permeability
    • Dilation of venules and capillaries
    • Different from urticaria
      • Limited to the superficial dermis
  • Generally resolves over 24-48 hours
  • Mast Cell / Kinin related etiologies
  • Involvement of the lips, pharynx and bowel common (potentially life-threatening)
  • Treated with CCS and H1/H2 blockers
    • FFP has been used in C1-Inh deficiency
etiology
Etiology
  • Immunologic / IgE mediated
    • elicited by foods, additives, drugs, transfusions, insect bites and infection
      • Release of vasoactive mediators from mast cells
      • Urticaria and pruritis
  • Hereditary and Acquired (non-mast cell)
    • Bradykinin and complement-mediated increases in vasc perm.
    • ACE-I or NSAID
ace inhibitors
Ace-Inhibitors
  • ACE (Kininase II) degrades bradykinin
    • Vasodilator
  • ACE-I results in inc levels bradykinin
    • Vasc B-2 rec’s for vasodilation, inc perm, inc NO
    • Not related to ACE-I induced cough
  • Pts with genetic deficiencies in bradykinin degradation could be at higher risk
  • 0.1-0.7% of patients tx with ACE-I
  • Intestinal edema may develop
    • Sudden onset abdom pain, vomiting and diarrhea
ace i
ACE-I
  • 50% of cases occur within 1 week of tx
    • Others may occur years later
  • Class specific
  • ARB’s - have been linked to episodes of angioedema
    • 2 of 26 pts with ACE-I induced angioedema persisted with an ARB and resolved with its withdrawal
    • Switch with caution
hereditary acquired
Hereditary/Acquired
  • Kinin-mediated angioedema that may be unmasked with use of ACE-I
  • C1-Inh deficiency (level or fx) - Inc BKinin
    • Normally fx to degrade C1 and prevent excessive complement activation
    • Dec levels of C1, C2, C4
    • Acquired- malignancy or auto-antibodies (older onset)
summary
Summary
  • Angioedema is potentially life-threatening
  • Associated pruritis and hives points to anaphylaxis
  • Absence of pruritis and hives think ACE-I and/or C1-Inh deficiency
    • PMHx, FHx, Malignancy
  • Use caution when switching from an ACE-I to an ARB
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